QDPR Anticuerpo(H-234): sc-98349

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QDPR (quinoid dihydropteridine reductase), also known as DHPR (dihydropteridine reductasae) or PKU2, is a member of the short-chain dehydrogenases/reductase (SDR) family of enzymes. Functioning as a homodimer, QDPR plays an important role in the recycling of tetrahydrobiopterin (BH4), an essential cofactor for the hydroxylation of the aromatic amino acids (tryptophan, tyrosine and phenylalanine). More specifically, QDPR catalyzes the regeneration of BH4 from quinonoid dihydrobiopterin (qBH2), the product generated from the hydroxylation reactions. Mutations in the gene encoding QDPR can lead to phenylketonuria II (also called PK2 or dihydropteridine reductase deficiency), a disorder resulting from the depletion of dopamine, epinephrine and serotonin due to defective recycling of BH4. Symptoms of PK2 include hyperphenylalaninemia, axial hypotonia, truncal hypertonia, microcephaly and abnormal thermogenesis.